Hirschsprung disease – Primer (2023) Louise Montalva

Description

Hirschsprung disease (HSCR) is a rare congenital intestinal disease characterized by the absence of ganglion cells in the intestine. Patients typically present in the neonatal period. Diagnosis is confirmed by rectal biopsy, and management involves surgical removal of the aganglionic bowel using procedures like Swenson, Soave, or Duhamel.